Chelating principles in Menkes and Wilson diseases

Role of PTA in the prevention of Cu(amyloid-β) induced ROS formation and amyloid-β oligomerisation in the presence of Zn

“…Cu dysmetabolism was also reported in other neurological diseases like prion diseases, motor neuron diseases and Parkinson’s disease 24–25. Cu is an essential metal ion26 and, in the case of neurons, this is strengthened by the fact that neurological disturbances are hallmarks of the congenital diseases of Cu metabolism, such Menkes’ (accumulation of Cu) and Wilson’s (lack of Cu) 27. Furthermore, it was found that, when in the synaptic cleft, Cu can control the activity of neurotransmitter receptors, thus affecting excitability.…”

A Review on Coordination Properties of Thiol-Containing Chelating Agents Towards Mercury, Cadmium, and Lead

“…Penicillamine’s distribution volume consists primarily of the extracellular space. Accidentally, penicillamine may give rise to serious adverse effects [157]. Also, d -penicillamine has been used as an antidote in Hg and Pb poisonings, before DMSA and DMPS were clinically introduced [130].…”

Current Biomedical Use of Copper Chelation Therapy

“…In a comparative clinical trial, a clear reduction of the number of patients with neurodegenerative disease in the group treated with TM was determined with respect to the TETA treated group [40]. Despite the potential efficiency and limited toxicity, the clinical use of TM is limited by instability of the ammonium formulation [4] and to low compliance due to frequency of dosing (6 times/day). For these limitations, a derivative of TM, the bis-choline-tetrathiomolybdate, has been recently introduced and a new multicenter phase II study has been performed, demonstrating the efficiency of the drug with no cases of paradoxical drug-related neurological worsening [41].…”

“…Copper-binding proteins include cytochrome oxidase, copper-zinc-superoxide dismutase, lysyl oxidase, tyrosinase, and dopamine-beta-monooxygenase, which are involved in pivotal biological processes like mitochondrial respiration, antioxidant defense, extracellular matrix cross-linking, pigmentation and neurotransmitter biosynthesis, respectively [2,3]. For an accurate list of copper-requiring enzymes, with particular emphasis on enzymes involved in genetic disorders of copper homeostasis, refer to Horn et al [4]. The majority of copper in the body is located in organs with high metabolic activity, such as liver, kidneys, heart and brain; approximately 5% of total copper is in the serum-of which, up to 95% is bound to ceruloplasmin (Cp).…”